sickle cell disease

Done by: nursing institutes fourms

Evaluated by: prince of nursing

Patient history

Mr. xxxx admitted to the male medical ward on xx/xx/xxxx complaining of severe pain in the back, L leg and chest.

He is 26 years old, male, Omani and single live in Bahla. He is student in the 9th class.

Vital signs was taking and showed normal value T: 36 C P: 80 beats/min, BP:110/70 mmHg, R: 20 breath/min. He had no fever, cough, or truma.

Patient is a known case of sickle sell anemia since he was a child .

He is admitted to the hospital every 2 to 3 weeks.

Past medical history:

The pt known case of SCD since child hood and known case of epileptic since 2 years. He had history of frequent admission with VOC and on regular exchange transfusion. Last admission in 5/2007 with ACS and treated in ICU.

Present medical history:

The pt was brought by his friends to the hospital on 26/9/2007 with the complaints of fits and with loss of consciousness. He is also present with back and LL pain. Given PHENERGAN and improve slightly. No H/O incontinence disturbance, no chest pain, no cough no fever and no H/O trauma.

Definition of the DiseaseDefinition of the Disease

Sickle sell anemia is a sever hemolytic anemia that result from inheritance of the sickle hemoglobin (HbS) gene. In hemolytic anemia, the red blood cells have shortened life span; thus, the number of the red blood cells in the circulation is reduced result in decreased available oxygen.

Pathophysiology

The sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change; consequently, the red blood cell containing (HbS) loses its round, very pliable, biconcave disk shape and become deformed, rigid, and sickle-shaped. These long, rigid red blood cells can adhere to the endothelium of the small vessel; when they pile up against each other, blood flow to a region or an organ may be reduced. If ischemia or infarction results, the patient may have pain, swelling, and fever.

The deformed red blood cells obstructs blood flow to tissue (vaso-occlusive crisis) joint pain, organ infarction and fatigue results due to hypoxia eventually. Virtually any organ may be affected by this, but the primary sites involve those areas with slowed circulation, such as the spleen, lungs, and central nervous system. Complications of the sickle cell anemia include infection, stroke, renal failure, heart failure, and pulmonary hypertension.

Etiology of the Disease:Etiology of the Disease:

According to bookAccording to book

--Gene Disorder (HbS).Gene Disorder (HbS).-Black people.-Black people.-Nutrition deficiency.-Nutrition deficiency.-Infection.-Infection.-Cardiac dysfunction.-Cardiac dysfunction.-Impaired production of -Impaired production of

RBC.RBC.-Decrease destruction -Decrease destruction

of RBC.of RBC.

In the patient In the patient

- - Patient have the Patient have the disease and 1 of his disease and 1 of his brotherbrother..

Clinical Manifestations of the Disease:

According to book According to book

Anemia with hemoglobin Anemia with hemoglobin value (7-10 g/dl)value (7-10 g/dl)

PalePale

TachycardiaTachycardia

Cardiac murmursCardiac murmurs

Enlarged heart Enlarged heart

In the patientIn the patient

Patient has (9.54 g/dl).Patient has (9.54 g/dl). The pt looks pale.The pt looks pale. The patient pulse was 80 The patient pulse was 80

beats/min.beats/min. Patient hadn’t cardiac Patient hadn’t cardiac

murmur.murmur.

Patient hadn’t enlarged Patient hadn’t enlarged heart.heart.

DysrhythmiaDysrhythmia Heart failure Heart failure Enlargement of the Enlargement of the

bone of the face and bone of the face and skullskull

Joint painJoint pain

Organ infarctionOrgan infarction

fatiguefatigue

Patient pulse was Patient pulse was regularregular

Patient hadn’t heart Patient hadn’t heart failurefailure

Patient face is normalPatient face is normal Patient was having Patient was having

joint pain when joint pain when admittedadmitted

Patient had no organ Patient had no organ infarctioninfarction

Patient was not very Patient was not very fatiguefatigue

CBC TestCBC Test

TestValueRangeRemarks

WBC15. 3 K/uL4-11*H

Neutrophils#6 .79 K/uL2-7.5*N

Lymphocytes#7 ..21 K/uL1.5-4.5*H

Monocytes#1 .05 K/uL0.2-0.8*H

Eosinophils#.105 K/uL0-0.4*N

Basophils#.155 K/uL0-0.2*N

RBC4.32K/uL4.5-6.5*L

Hemoglobin12.1 G/DL13-17*L

Hematocrit34..2 %40-52*L

Mean Corpuscular Volume(MCV)

79.2 FL74-95*N

Mean Corpuscular Hemoglobin(MCH)

28.1 PG26-32*N

Mean Corpuscular Hemoglobin Centration(MCHC)

35.4 G/DL31.5-36*N

Platelet #454 K/uL150-400*H

RDW26 % CV11-14*H

Mean Plasma Volume (MPV)10.2 FL7.8-11*N

Reticlocyte Reticlocyte countcount

5.25.20.2-20.2-2**HH

Treatment

Non-Pharmacological

•Encourage the patient to use relaxation techniques.

•Teach the patient breathing exercise.

•Apply moist heat to painful areas.

•Position the patient in comfortable position.

Pharmacological

Name of Name of DrugDrug

classificationclassificationRouteRouteDoseDoseFrequencyFrequency

Folic AcicFolic AcicFolic acidFolic acidOralOral5mg5mgODOD

ParacetamoParacetamoll

AntipyreticAntipyretic

AnalgesicAnalgesic

OralOral10001000 mgmg

SOSSOS

HydroxyureHydroxyureaa

AntineoplasiAntineoplasic agentc agent

OralOral500m500mgg

BIDBID

Sodium Sodium valproatevalproate

))NSAIDNSAID ( (analgesic analgesic

(non-(non-narcotic)narcotic)

OralOral500m500mgg

TIDTID

Folic acid:

Action

-Treatment of megoblastic anemia due to nutritional deficiency, infection, and childhood

-Required for nucleoprotien synthesis and maintenance of normal erythropoisis.

Possible Side Effects

- Hypersensitivity: Allergic reaction.

- Local: pain and discomfort.

Nurse’s Responsibilities•Monitor patient for hypersensitivity reaction, specially if drug previously taken.

•Test using schilling test and serum vitamin B12 levels to rule out pernicious anemia.

•Report rash, difficulty breathing, pain or discomfort.

Paracetamol

Action - Inhibits the synthesis of prostaglandis in the CNS and peripherally blocks pain impuls generation.

- Artipyresis from inhibition of hypothalamus heat regulating center.

Possible side effects•CNS: Dizziness, insomnia, fatigue.•GI: nausea, dyspepsia, GI pain, vomiting.•Respiratory: dyspnea, bronchospasm, rhinitis•Hematologic: bleeding, aplastic anemia, platelet inhibtion when higher dose.

Nurse’s responsibility:

•Take the drug only of complaint indicated, not on anti inflammatory agent.

•Avoid the use of other OTC preparation. They may contain acetaminophen and serious overdose can occur.

- Report skin rash, unusual bleeding or bruising, yeiioe of the skin or eyes.

Hydroxyurea

Action

- Inhibits an enzyme that is crucial for DNA synthesis.

- Use to reduce frequency of painful crises and to reduce the need for blood transfusions in adult patient with sickle cell anemia.

Possible side effects

•CNS: sedation, dizziness, headache, confusing, sweating, seizures.

•GI: nausea, vomiting, dry mouth, constipation, flatulance.

Nurse’s responsibility

•Prepare a calendar for dates to return for diagnostic testing and treatment days.

•Arrange for regular blood tests to monitor the drug's effect.

• Inform the pt to drink at least 10-12 glasses of water while on this drug.

• Report fever, chills, sore throat, unusual bleeding or severe nausea and vomiting.

Nursing Care Plane:

Nursing diagnosis:

Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels as manifested by patient verbalize of pain and discomfort.

Goal:

To reduce pain.

Nursing interventionsRationale Rationale

Assess severity and Assess severity and location of painlocation of painEncourage oral intake of Encourage oral intake of fluids and monitor intake fluids and monitor intake and output.and output.Teach and encourage use Teach and encourage use of non-pharmacological of non-pharmacological treatment.treatment.Administer analgesic as Administer analgesic as prescribed. prescribed. Provide support and Provide support and carefully position affected carefully position affected extremities.extremities.

Tissue and organ are Tissue and organ are susceptible to hypoxic. susceptible to hypoxic. damage which cause pain.damage which cause pain.To promote hemodilution To promote hemodilution and reverse agglutination and reverse agglutination process.process.Joint pain can be Joint pain can be minimized during crisis by minimized during crisis by this way.this way.To relief the pain. To relief the pain. Reduce edema, Reduce edema, discomfort and risk of discomfort and risk of injury especially with injury especially with osteomylititis. osteomylititis.

Outcomes

The patient:

•Identifies the importance of non-pharmacological treatment and uses it.

•Increases fluid intake.

•Verbalizes that pain is relief after administration of analgesic.

Nursing Diagnosis

Risk for infection related to altered immunologic response as manifested by changes in the WBC count.

Goal

To prevent infection.

Nursing interventionsRationale Rationale Assess for sign and Assess for sign and symptoms of infection.symptoms of infection.Encourage early Encourage early ambulation and pulmonary ambulation and pulmonary hygiene.hygiene.Promote adequate Promote adequate nutrition and fluid intake.nutrition and fluid intake.Monitor pt for signs of Monitor pt for signs of infection e.g. fever and infection e.g. fever and increase WBC.increase WBC.Monitor vital signs.Monitor vital signs.

The physiological stress The physiological stress result from infection often result from infection often precipitates crisisprecipitates crisisActivity mobilizes Activity mobilizes pulmonary secretions.pulmonary secretions.Optimal nutrition and Optimal nutrition and fluid balance can promote fluid balance can promote the body immunologic the body immunologic

responseresponse..To initiate treatment as To initiate treatment as soon as possible.soon as possible.To prevent more To prevent more serious complication.serious complication.

Outcomes

The patient:

•Has clear breath sound.

•Has WBCs within normal range(5000-10000 cu mm).

•Has negative result of sputum cultures.

•Pt report no signs and symptoms of infection.

Discharge Plan:

-MR. xxxx 26 years old male, single, Omani. Pt admitted to the M.M.W on xx/xx/xxxx at 17:00 PM complained of back and LL pain. He is known case of sickle cell disease brought by his friends with back and LL pain. The pt had no history of incontinence disturbance, no fever, no cough and no trauma.

Discharge condition:

- The pt discharge on xx/xx/xxxx at 11:37 AM. Case of SCD, epilepsy admitted for exchange transfusion which was done after topping up BH levels.

- Post transfusions HB levels acceptable, discharge state improved.

PT is on medication:

Drug NameDrug NameRouteRouteDoseDose DaysDays

- -Folic AcidFolic Acid

--ParacetamolParacetamol

--HydroxeureaHydroxeurea

--Sodium Sodium ValproateValproate..

OralOral

OralOral

OralOral

OralOral

55 MG MG ODOD

500500 MG MG SOSSOS 500 MG 500 MG BIDBID

500 MG 500 MG TIDTID

3535

3535

3535

3535

Discharge advice / Health education:

•Follow up in medicine clinic in NPC.

•Avoid exposure to very hot or very cold environment.

•Use the diversion activities (breathing exercise, relaxation technique and careful movement) to minimize the pain.

•Avoid stressful situation and hard activities (plying football) because it can lead to sickle crisis.

•Take food that including liver, green leafy vegetables and citrus food, wheat germ and regarding supplementary vitamins like Folic acid.